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Multicenter Study
. 2023 Jun;25(6):845-853.
doi: 10.1002/ejhf.2823. Epub 2023 Mar 15.

Diagnostic pathways to wild-type transthyretin amyloid cardiomyopathy: a multicentre network study

Giacomo Tini  1   2 , Paolo Milani  3   4 , Mattia Zampieri  5 , Angelo G Caponetti  6   7 , Francesca Fabris  3   4 , Andrea Foli  4 , Alessia Argirò  5 , Carlotta Mazzoni  5 , Christian Gagliardi  6   8 , Simone Longhi  6   8 , Giulia Saturi  6   7 , Giuseppe Vergaro  9   10 , Alberto Aimo  9   10 , Domitilla Russo  2 , Guerino G Varrà  11 , Matteo Serenelli  12 , Gioele Fabbri  12 , Laura De Michieli  13 , Giuseppe Palmiero  14 , Giuseppe Ciliberti  15   16 , Samuela Carigi  17 , Eugenio Sessarego  1 , Giulia E Mandoli  18 , Giulia Ricci Lucchi  19 , Valeria Rella  20 , Enrico Monti  21 , Elisa Gardini  21 , Michela Bartolotti  22 , Lia Crotti  20   23 , Elisa Merli  19 , Roberta Mussinelli  4 , Pier Filippo Vianello  1 , Matteo Cameli  18 , Francesca Marzo  17 , Federico Guerra  15   16 , Giuseppe Limongelli  8   14 , Alberto Cipriani  13   24 , Stefano Perlini  3   4 , Laura Obici  4 , Federico Perfetto  5 , Camillo Autore  2   25 , Italo Porto  1   26 , Claudio Rapezzi  12   27 , Gianfranco Sinagra  8   11 , Marco Merlo  8   11 , Beatrice Musumeci  2 , Michele Emdin  9   10 , Elena Biagini  6   8 , Francesco Cappelli  5 , Giovanni Palladini  3   4 , Marco Canepa  1   26
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Free article
Multicenter Study

Diagnostic pathways to wild-type transthyretin amyloid cardiomyopathy: a multicentre network study

Giacomo Tini et al. Eur J Heart Fail. 2023 Jun.
Free article

Abstract

Aim: Epidemiology of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) remains poorly defined. A better characterization of pathways leading to ATTRwt-CA diagnosis is of key importance, and potentially informative of disease course and prognosis. The aim of this study was to describe the characteristics of contemporary pathways leading to ATTRwt-CA diagnosis, and their potential association with survival.

Methods and results: This was a retrospective study of patients diagnosed with ATTRwt-CA at 17 Italian referral centres for CA. Patients were categorized into different 'pathways' according to the medical reason that triggered the diagnosis of ATTRwt-CA (hypertrophic cardiomyopathy [HCM] pathway, heart failure [HF] pathway, incidental imaging or incidental clinical pathway). Prognosis was investigated with all-cause mortality as endpoint. Overall, 1281 ATTRwt-CA patients were included in the study. The diagnostic pathway leading to ATTRwt-CA diagnosis was HCM in 7% of patients, HF in 51%, incidental imaging in 23%, incidental clinical in 19%. Patients in the HF pathway, as compared to the others, were older and had a greater prevalence of New York Heart Association (NYHA) class III-IV and chronic kidney disease. Survival was significantly worse in the HF versus other pathways, but similar among the three others. In multivariate model, older age at diagnosis, NYHA class III-IV and some comorbidities but not the HF pathway were independently associated with worse survival.

Conclusions: Half of contemporary ATTRwt-CA diagnoses occur in a HF setting. These patients had worse clinical profile and outcome than those diagnosed either due to suspected HCM or incidentally, although prognosis remained primarily related to age, NYHA functional class and comorbidities rather than the diagnostic pathway itself.

Keywords: Cardiac amyloidosis; Epidemiology of cardiac amyloidosis; Tafamidis; Wild-type transthyretin cardiac amyloidosis.

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References

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